Autopsy findings of co-sleeping-associated sudden unexpected deaths in infancy: relationship between pathological features and asphyxial mode of death.

AIM: Co-sleeping is associated with increased risk of sudden unexpected death in infancy (SUDI)/sudden infant death syndrome (SIDS). The aim of this study is to examine autopsy findings from a single U.K. specialist centre to determine the relationship between co-sleeping and cause of death. METHODS: Retrospective analysis of >1500 paediatric autopsies carried out by paediatric pathologists over a 10-year period. SUDI was defined as sudden unexpected death of an infant aged 7-365 days; deaths were categorised into explained SUDI (cause of death was determined) and unexplained SUDI (equivalent to SIDS). RESULTS: There were 546 SUDI; sleeping arrangements were specifically recorded in 314; of these, 174 (55%) were co-sleeping-associated deaths. Almost two thirds (59%) of unexplained SUDI were co-sleeping compared to 44% explained SUDI (95% confidence interval (CI) 1.0-27.2%, P=0.03); however, this difference remained statistically significant only for the first 5 months of life (95% CI 3.5-33.2%, P=0.01). In unexplained SUDI aged < 6 months, there were no significant differences between co-sleeping and non-co-sleeping deaths with respect to ante-mortem symptoms, intrathoracic petechiae, macroscopic lung appearances, pulmonary haemosiderin-laden macrophages, and isolation of specific bacterial pathogens; however, fresh intra-alveolar haemorrhage was reported more commonly in co-sleeping (54%) than in those that were not (38%; 95% CI 1.4-30.5%, P=0.03). CONCLUSIONS: Co-sleeping is associated with unexplained SUDI/SIDS in infants aged < 6 months, suggesting that co-sleeping is related to the pathogenesis of death in younger infants. The finding that intra-alveolar haemorrhage is more common in co-sleeping suggests that a minority of co-sleeping-associated deaths may be related to an asphyxial process.

Sudden unexpected neonatal death in the first week of life: autopsy findings from a specialist centre.

OBJECTIVE: Sudden unexpected early neonatal death (SUEND) in the first week of life shares features with sudden unexpected death in infancy (SUDI) but is not included as SUDI, which is limited to post-perinatal deaths. The aim of this study was to review SUEND autopsies performed in a single specialist centre over a 10-year period, (1996-2005). METHODS: Retrospective analysis of >1500 consecutively performed paediatric autopsies performed by paediatric pathologists at one centre conducted according to a standard protocol including ancillary investigations. SUENDs were identified and autopsy findings reviewed. RESULTS: Of 1516 post-mortem examinations, 180 were first-week neonatal deaths, 55 (31%) presenting as SUEND. Thirty-two (58%) were explained following autopsy, whilst the remainder were unexplained; most deaths during sleep were associated with adult co-sleeping. Around 40% of explained deaths were associated with undiagnosed congenital abnormalities, mainly congenital heart disease. In addition, there were nine infection-related deaths and three deaths from unsuspected metabolic disease (fatty acid oxidation defects). CONCLUSION: There are distinct differences between SUEND and SUDI, with significantly more explained deaths in the former and a much greater proportion due to congenital abnormalities and metabolic disease.

Rib fractures identified at post-mortem examination in sudden unexpected deaths in infancy (SUDI).

Rib fractures may be associated with non-accidental injury (NAI) in infancy, but the possible significance of fresh rib fractures in relation to resuscitation remains undetermined. Consequently, it is important to detect and confirm the presence of rib fractures when performing a post-mortem examination, particularly in the context of sudden unexpected death in infancy (SUDI). At our centre, it has been local policy to perform routine radiological skeletal surveys and detailed post-mortem examination of the ribs in all SUDI autopsies. The aim of this study is to establish the characteristics of all rib fractures identified at autopsy in the setting of SUDI from a large series of cases examined at a single specialist centre. As part of a larger review of paediatric post-mortem examinations performed at a single specialist institution over a 10-year period (1996-2005), all cases presenting as SUDI (aged 7-365 days) were identified and their anonymised records searched to identify all cases in which rib fractures were recorded. Over the 10-year period, 546 post-mortem examinations were performed for the indication of SUDI, including 94 forensic autopsies. Rib fractures were identified in 24 cases (4%). 15 infants (3% of SUDI) demonstrated healing rib fractures, of which 10 infants (67%) showed additional features suggestive of NAI. The other 9 infants (2% of SUDI) demonstrated fresh rib fractures only with no surrounding tissue reaction histologically; in 7 (78%) of these there were no other injuries and the fresh fractures were interpreted to have been caused by resuscitation-related trauma. All of the resuscitation-related fractures were situated in the anterolateral chest, in contrast to NAI-associated fractures, which were located in the anterolateral and/or posterior chest. Anterior costochondral junction fractures were also seen in a minority of NAI-associated cases, but such fractures were not seen in apparent resuscitation-related cases. Compared to healing rib fractures, which were detected on skeletal survey in 93%, fresh rib fractures were only detected in 22% of skeletal surveys. Rib fractures are uncommon in infancy and may indicate NAI, particularly when healed or healing, posterior or involving the costochondral junction. Fresh rib fractures may be missed on skeletal survey, but can be reliably detected at post-mortem examination following stripping of the pleura and detailed examination of each rib. Fresh anterolateral fractures, which may be multiple, contiguous and even bilateral, are highly likely to be related to resuscitation if there are no other associated injuries.

Predictors of long-term outcome of children with idiopathic focal segmental glomerulosclerosis.

Clinical and histological data of children presenting with steroid-resistant nephrotic syndrome and renal biopsy showing focal and segmental glomerulosclerosis from 1980 with a follow-up of over 10 years were reviewed. There were 66 patients; 38 male and 28 female. Age at onset ranged from 0.4-14.1 years (mean 6.4). Tubular atrophy was present at first biopsy in 50/66, capsular adhesions in 35/66, glomerular tip lesions in 8/66 and mesangial expansion in 31/66 patients. In 51 children, cyclophosphamide was prescribed as the first cytotoxic agent, while 15 received cyclosporine A and complete remission was induced in 43 and 40% of the children, respectively. Complete and stable remission was maintained in 35 children, while 22 had reduction of proteinuria with symptomatic relief. Nine were refractory to cytotoxic therapy. Of the 35 patients who entered complete and stable remission, the renal survival was over 90%, while in the 31 non-responders it was 48% in 10 years. The multivariate analysis using unconditional logistic regression method identified the presence of mesangial expansion (p=0.011) and tip lesions (p=0.005) as the independent predictors of favourable response to cytotoxic therapy and the presence of renal impairment (p=0.008) and extensive focal segmental sclerosis (p=0.025) as independent predictors of unfavourable response.

Childhood thin GBM disease: review of 22 children with family studies and long-term follow-up.

Thin glomerular basement membrane (GBM) disease is generally known to have a good renal prognosis, although renal insufficiency has sometimes been reported and the overlap with Alport syndrome implies that a good prognosis cannot be guaranteed. In order to shed light on long-term prognosis of thin GBM disease we have retrospectively evaluated 22 children with persistent haematuria and biopsy-proven thin GBM. Mean follow up was 7 years (range 2-17 years), mean age at onset was 7 years (range 1.5-15). Biopsies were performed a mean of 3.8 years after detection of hematuria. The light microscopy (LM) and immunofluorescence (IF) findings were essentially unremarkable in all of the children, while electron microscopy (EM) showed thinning of the GBM in all cases and no changes characteristic of Alport syndrome. The family history was positive for renal disease in 17 (77.3%) patients with hematuria in 8 (36.3%) families, and hematuria with renal failure (RF) or deafness in 9 (40.9%). It was completely negative for renal disease in 4 (18.2%) and unavailable in 1 (4.5%). Four patients (18%) showed a decline in renal function after 6, 8, 9 and 12 years of follow-up, and 1 of these also developed hearing impairment. None developed hypertension. Our study suggests that thin GBM disease is not always benign and a child with thin GBM should never be assigned such a prognosis, especially if there is a family history of renal impairment or deafness, where careful follow-up is needed due to the risk of late onset renal failure.